Department of Pediatric Cardiology, West China Second University Hospital, Sichuan University, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, Sichuan, China.
Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu, Sichuan, China.
Pediatr Rheumatol Online J. 2020 Jul 14;18(1):61. doi: 10.1186/s12969-020-00452-7.
Kawasaki disease (KD) is an acute, self-limiting systemic vasculitis that predominately affects children. Neurological involvement is a known complication of KD, however, its association with KD severity remains elusive. We aimed to systematically describe the general manifestations of neurological involvement in KD, determine whether neurological involvement is a marker of disease severity in patients with KD, and assess the relationship of such involvement with intravenous immunoglobulin (IVIG) resistance and coronary artery lesions (CALs).
We retrospectively reviewed data from 1582 patients with KD between January 2013 and December 2017. Profiles of patients with neurological symptoms (group A, n = 80) were compared to those of gender- and admission date-matched patients without neurological involvement (group B, n = 512). Multivariate logistic regression analyses were performed to determine whether neurological involvement was significantly associated with IVIG resistance.
Neurological involvement was observed in 5.1% (80/1582) of patients with KD. The neurological manifestations were diffuse, presenting as headache (13/80, 16.3%), convulsions (14/80, 17.5%), somnolence (40/80, 50.1%), extreme irritability (21/80, 26.3%), signs of meningeal irritation (15/80, 18.8%), bulging fontanelles (7/80, 8.8%), and facial palsy (1/80, 1.3%). Neurological symptoms represented the initial and/or predominant manifestation in 47.5% (38/80) of patients with KD. The incidence of IVIG resistance and levels of inflammatory markers were higher in group A than in group B. However, neurological involvement was not an independent risk factor for IVIG resistance or CALs.
Rates of neurological involvement were relatively low in patients with KD. Neurological involvement was associated with an increased risk of IVIG resistance and severe inflammatory burden. Our results highlight the need for pediatricians to recognize KD with neurological involvement and the importance of standard IVIG therapy.
Retrospectively registered.
川崎病（KD）是一种主要影响儿童的急性、自限性全身性血管炎。神经系统受累是 KD 的已知并发症，然而，其与 KD 严重程度的关系仍不清楚。我们旨在系统描述 KD 中神经系统受累的一般表现，确定神经系统受累是否是 KD 患者疾病严重程度的标志物，并评估其与静脉注射免疫球蛋白（IVIG）耐药和冠状动脉病变（CALs）的关系。
我们回顾性分析了 2013 年 1 月至 2017 年 12 月间 1582 例 KD 患者的数据。将有神经系统症状的患者（A 组，n=80）的特征与性别和入院日期相匹配的无神经系统受累患者（B 组，n=512）进行比较。进行多变量 logistic 回归分析，以确定神经系统受累是否与 IVIG 耐药有显著关联。
80/1582 例 KD 患者（5.1%）出现神经系统受累。神经系统表现为弥漫性，表现为头痛（13/80，16.3%）、抽搐（14/80，17.5%）、嗜睡（40/80，50.1%）、极度烦躁（21/80，26.3%）、脑膜刺激征（15/80，18.8%）、囟门膨隆（7/80，8.8%）和面瘫（1/80，1.3%）。KD 患者中，47.5%（38/80）的神经系统症状是首发和/或主要表现。A 组患者的 IVIG 耐药发生率和炎症标志物水平均高于 B 组。然而，神经系统受累并不是 IVIG 耐药或 CALs 的独立危险因素。
KD 患者的神经系统受累发生率相对较低。神经系统受累与 IVIG 耐药和严重炎症负担的风险增加有关。我们的研究结果强调了儿科医生识别 KD 合并神经系统受累的必要性，以及标准 IVIG 治疗的重要性。
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